Longstanding cobblestone plaque in the lumbar region of a 13-year-old boy

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A 13-year-old boy presented with a 10-year history of a gradually enlarging but otherwise asymptomatic lesion on his back. He was otherwise well with no relevant family history. On physical examination, a yellowish plaque, 100 9 70 mm in size, with a cobblestone surface, was seen on the patient’s right lumbar region (Fig. 1). Radiography showed no evidence of osteopoikilosis to suggest Busche–Ollendorf syndrome. The clinical differential diagnosis included connective-tissue naevus, sebaceous naevus and plaque-type juvenile xanthogranuloma. An incisional skin biopsy was taken.